Long-term cognitive outcome in adult survivors of an early childhood posterior fossa brain tumour

Abstract

Purpose Posterior fossa brain tumours (PFT) and their treatment in young children are often associated with subsequent cognitive impairment. However, reported follow-up periods rarely exceed 10 years. This study reports very long-term cognitive consequences of surviving an early childhood PFT. Methods 62 adult survivors of a PFT, ascertained from a national register, diagnosed before 5 years of age, and a sibling control, received a single IQ assessment an average of 32 years (range 18–53) after initial diagnosis, using the Weschler Abbreviated Scale of Intelligence. Regression models were fitted to survivor–sibling pair differences on verbal and performance IQ (VIQ and PIQ) scores to investigate whether increasing time between PFT diagnosis and follow-up IQ assessment contributed to survivor–sibling IQ differences. Results At follow-up, survivors had, on average, VIQ 15 points and PIQ 19 points lower than their siblings. There was no significant effect of time since diagnosis on survivor–sibling VIQ difference. Survivors who received radiotherapy showed no significant effect of time since diagnosis on survivor–sibling PIQ difference. Survivors who did not receive radiotherapy demonstrated a trend for it to reduce. Conclusions VIQ and PIQ deficits persist in adulthood, suggesting the effect of a fixed injury imposing on cognitive development, rather than an ongoing pathological process. Implications for cancer survivors The findings will help parents and others supporting survivors of an early life PFT to identify and plan for possible cognitive outcomes, and highlight the importance of early interventions to optimize cognitive function during the developmental period.

Abnormal Cerebellar Development in Autism Spectrum Disorders

“…PFS is characterized by a similar but often more severe constellation of symptoms including mutism, emotional lability, ataxia, hypotonia, and behavioral disturbances [28,29]. While the acute, severe presentation of PFS often resolves with time, children who experience PFS often suffer long-term neurocognitive impairment [30][31][32]. The presence of linguistic, cognitive, and behavioral deficits in patients with CCAS and PFS may further implicate disruptions of cerebellar function in ASD, which is a pervasive developmental disorder characterized by an earlier onset and broader spectrum of symptom severity in these domains.…”

Randomized clinical trial on the effects of a computerized cognitive training for pediatric patients with acquired brain injury or congenital malformation

“…From a cognitive point of view, grey and white matter anomalies of the brain may cause impairments in global intelligence as well as in core cognitive domains, such as attention, memory, executive functions, processing speed and visual–spatial abilities 5 , 15 – 17 . Also, cerebellar alterations, either in acquired or congenital diseases, may affect cognition and emotions, not limiting their effects on the motor domain 18 – 24 .…”

Computer-Based Cognitive Training in Children with Primary Brain Tumours: A Systematic Review

“…The most common cognitive disorders are visuo-spatial reasoning, motor functioning, attention, working memory, processing speed, and executive functioning [ 2 , 3 ]. In addition, some deficits in socio-cognitive skills have also emerged due to the long-term effects of childhood cancer, such as isolation, difficulty solving social problems, relationship problems, and peer rejection [ 3 ].…”

“…The most common cognitive disorders are visuo-spatial reasoning, motor functioning, attention, working memory, processing speed, and executive functioning [ 2 , 3 ]. In addition, some deficits in socio-cognitive skills have also emerged due to the long-term effects of childhood cancer, such as isolation, difficulty solving social problems, relationship problems, and peer rejection [ 3 ]. Poor quality of life in survivors of paediatric BTs was reported by Mostow et al [ 4 ] who examined 342 adults who had been treated for brain tumours before the age of 20 and who had survived at least 5 years.…”